Posterior quadrant disconnection surgery for <scp>S</scp>turge‐<scp>W</scp>eber syndrome

<jats:title>Summary</jats:title><jats:sec><jats:title>Objective</jats:title><jats:p>Some patients with <jats:styled-content style="fixed-case">S</jats:styled-content>turge‐<jats:styled-content style="fixed-case">W</jats:styled-content>eber syndrome (<jats:styled-content style="fixed-case">SWS</jats:styled-content>) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. The majority of patients with <jats:styled-content style="fixed-case">SWS</jats:styled-content> have leptomeningeal angioma located over the temporal, parietal, and occipital lobes. We applied posterior quadrant disconnection surgery for this type of <jats:styled-content style="fixed-case">SWS</jats:styled-content> with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Ten patients who were surgically treated using the posterior quadrantectomy (<jats:styled-content style="fixed-case">PQT</jats:styled-content>) were enrolled in this study. Surgical outcome was analyzed as seizure‐free or not at 2 years after surgery. Psychomotor development was evaluated by the scores of mental developmental index (<jats:styled-content style="fixed-case">MDI</jats:styled-content>) and psychomotor developmental index (<jats:styled-content style="fixed-case">PDI</jats:styled-content>) in the Bayley Scales of Infant Development <jats:styled-content style="fixed-case">II</jats:styled-content> preoperatively, and at 6 and 12 months after the <jats:styled-content style="fixed-case">PQT</jats:styled-content>.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>Eight of 10 patients were seizure‐free. Patients without complete elimination of the angiomatous areas had residual seizures. Average <jats:styled-content style="fixed-case">MDI</jats:styled-content> and <jats:styled-content style="fixed-case">PDI</jats:styled-content> scores before the surgery were 64.8 and 71.6, respectively. Scores of <jats:styled-content style="fixed-case">MDI</jats:styled-content> at 6 and 12 months after the <jats:styled-content style="fixed-case">PQT</jats:styled-content> in seizure‐free patients were 80.5 and 84.5, respectively (p < 0.01). <jats:styled-content style="fixed-case">PDI</jats:styled-content> scores at these postoperative intervals were 87.3 and 86.4, respectively (p < 0.05). Patients with residual seizures did not improve in either <jats:styled-content style="fixed-case">MDI</jats:styled-content> or <jats:styled-content style="fixed-case">PDI</jats:styled-content>.</jats:p></jats:sec><jats:sec><jats:title>Significance</jats:title><jats:p>The <jats:styled-content style="fixed-case">PQT</jats:styled-content> achieved good seizure control and improved psychomotor development in patients with <jats:styled-content style="fixed-case">SWS</jats:styled-content>. The complete deafferentation of angiomatous areas is required for seizure‐free results and psychomotor developmental improvement.</jats:p><jats:p>A PowerPoint slide summarizing this article is available for download in the Supporting Information section <jats:ext-link xmlns:xlink="http://www.w3.org/1999/xlink" xlink:href="http://onlinelibrary.wiley.com/doi/10.1111/epi.12547/supinfo">here</jats:ext-link>.</jats:p></jats:sec>